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Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS

The CCS copper chaperone is critical for maturation of Cu, Zn-superoxide dismutase (SOD1) through insertion of the copper co-factor and oxidization of an intra-subunit disulfide. The disulfide helps stabilize the SOD1 polypeptide, which can be particularly important in cases of amyotrophic lateral s...

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Detalles Bibliográficos
Main Authors: Proescher, Jody B., Son, Marjatta, Elliott, Jeffrey L., Culotta, Valeria C.
Formato: Artigo
Idioma:Inglês
Publicado: Oxford University Press 2008
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC2900889/
https://ncbi.nlm.nih.gov/pubmed/18337307
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddn063
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